Harries-Jones R, Knight R, Will R G, Cousens S, Smith P G, Matthews W B
University Department of Clinical Neurology, Radcliffe Infirmary, Oxford, UK.
J Neurol Neurosurg Psychiatry. 1988 Sep;51(9):1113-9. doi: 10.1136/jnnp.51.9.1113.
An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0.49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls.
研究人员试图确定1980年至1984年这5年间在英格兰和威尔士发生的所有克雅氏病病例。年平均死亡率为每百万人口0.49例；女性比男性更易患病。年龄别死亡率在70岁时达到峰值。一项病例对照研究涉及了122例确诊和疑似病例中的92例，该研究未能证实此前怀疑的环境致病因素的存在。尽管在病例对照研究中没有确诊的家族性克雅氏病病例，但近亲中的痴呆症明显比对照组更为常见。
